原发性浆细胞白血病与淋巴瘤伴浆细胞增多的诊断与鉴别诊断

发布时间：2018-04-15 11:20

本文选题：原发性浆细胞白血病 + 淋巴瘤　；参考：《临床与实验病理学杂志》2017年05期

【摘要】：目的探讨原发性浆细胞白血病(primary plasma cell leukemia,PPCL)及淋巴瘤伴浆细胞增多的临床病理特征、诊断及鉴别诊断。方法采用临床资料及细胞形态学、流式细胞术、免疫固定电泳及免疫组化Eli Vision两步法等检测7例PPCL及3例淋巴瘤伴浆细胞增多,并进行分析。结果 7例PPCL及3例淋巴瘤伴浆细胞增多的临床特点均以进行性贫血、血小板减少、发热、肝脾及淋巴结肿大最为常见;外周血细胞形态学分类浆细胞比例均大于20%,且伴形态学异常;外周血流式细胞免疫表型显示7例PPCL均表达CD38及CD138,2例表达CD56,2例表达CD20,轻链(Lamda、Kappa)均呈单克隆限制性表达,符合PPCL诊断;3例淋巴瘤伴浆细胞增多CD19、CD45呈弱阳性,CD38、CD138呈阳性,轻链Ig L未见限制性表达,属于正常浆细胞的免疫表型。3例轻链(Ig)未见限制性表达,经淋巴结切除活检病理学检查确诊血管免疫母细胞性T细胞淋巴瘤2例,CD30阳性窦内大B细胞淋巴瘤1例。结论 PPCL与淋巴瘤伴浆细胞增多有相同的临床表现及相似的细胞形态学特征,PPCL的诊断需结合免疫固定电泳及流式细胞免疫表型;而淋巴瘤伴浆细胞增多还需结合淋巴结组织学检查才能确诊。
[Abstract]:Objective to investigate the clinicopathological features, diagnosis and differential diagnosis of primary plasma cell leukemia-PPCL and lymphoma with plasmacytosis.Methods Clinical data, cell morphology, flow cytometry, immunofixation electrophoresis and immunohistochemical Eli Vision two-step method were used to detect 7 cases of PPCL and 3 cases of lymphoma with plasma cell proliferation.Results the clinical features of 7 cases of PPCL and 3 cases of lymphoma with plasmacytosis were progressive anemia, thrombocytopenia, fever, hepatosplenomegaly and lymphadenopathy.The percentage of plasma cells in peripheral blood cell morphological classification was more than 20 and accompanied by morphological abnormality, the expression of CD38 was detected in 7 cases of PPCL and 2 cases of CD56T in 2 cases of CD138T, and the expression of Lamda kappaa was restricted by monoclonal expression.According to PPCL diagnosis, CD19 + CD45 was weakly positive in 3 cases of lymphomas with plasmacytosis, CD38 + CD138 was positive, no restricted expression was found in light chain IgL, and no restricted expression was found in 3 cases of normal plasmacyte immunophenotype.One case of large B cell lymphoma with CD30 positive in sinus was confirmed by lymphadenectomy biopsy in 2 cases of angioblastoma.Conclusion PPCL and lymphoma with plasmacytosis have the same clinical manifestations and similar cellular morphological features. The diagnosis of PPCL should be combined with immunofixation electrophoresis and flow cytometry immunophenotype.However, lymphomas with plasmacytosis must be confirmed by histologic examination of lymph nodes.
【作者单位】：河北医科大学第四医院血液病实验室;
【基金】：河北省科技厅大健康服务和生物医药专项课题(162777243)
【分类号】：R733

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